Cardiovascular manifestations of mast cell disease: Part 4 of 5

Heart failure is uncommon in mast cell patients, but is noteworthy as a condition that involves mast cell activation.  One study of adults with SM found 12 patients out of 548 had congestive heart failure.  A small study with 18 MCAS patients found that persistent mast cell activation did not affect such parameters as systolic left ventricular function, systolic and diastolic left ventricular diameter, or shortening fraction.  These markers are often tied to heart failure. In that same study, 12/18 MCAS patients did exhibit a diastolic left ventricular dysfunction.  This defect is a sensitive indicator of changes to the myocardium, muscle around the heart and can be found using Doppler imaging. Five of those MCAS patients also showed hypertrophy in the left ventricle, a thickening of tissue that can be linked to heart damage.

Importantly, these findings were not linked to chronic heart failure in this population.  Mast cell patients should be aware that while these anatomical changes of the left ventricle may be present, there is not currently any indication that their increase the frequency of symptomatic heart failure in this population.  Mast cells are heavily involved in tissue remodeling and it is possible that local mast cell activation can lead to laying of additional tissue or scarring.  Tryptase, chymase and matrix metalloproteinases, all released by mast cells, participate in tissue remodeling and fibrosis.

Tryptase has been associated with both heart failure and atherosclerosis, involved in coronary disease and syndromes.  A number of other mediators can also contribute to heart failure, including histamine, platelet activating factor, IL-4, IL-6, IL-10, TNF, fibroblast growth factor (FGF) and transforming growth factor beta (TGFB).

Treatment of heart failure in mast cell patients is not terribly different from that of the general population.  Diuretics are often used first, including furosemide. Angiotensin receptor antagonists like losartan are good choices for mast cell patients since ACE inhibitors and beta blockers should be avoided wherever possible.  Calcium channel blockers like verapamil can be used. Spironolactone or similar medications may provide additional benefit. Ivabradine, a newer medication that works by affecting the funny current (Author’s note: Not a joke!  My favorite pathway name), is also a consideration.  Digoxin is appropriate for atrial fibrillation (afib) where other attempts to correct rhythm have failed.

References:

Kolck UW, et al. Cardiovascular symptoms in patients with systemic mast cell activation disease. Translation Research 2016; x:1-10.

Gonzalez-de-Olano D, et al. Mast cell-related disorders presenting with Kounis Syndrome. International Journal of Cardiology 2012: 161(1): 56-58.

Kennedy S, et al. Mast cells and vascular diseases. Pharmacology & Therapeutics 2013; 138: 53-65.

3 Responses

  1. Yvonne April 30, 2016 / 2:08 pm

    Hi Lisa,
    Thank you for these posts on heart disease and mast cell disease. I hope the medical community is taking note of your blog.

    Would you consider addressing the ingestion of oddball stuff like morning mouth goo, lipstick, and whatever else people inadvertantly swallow in the course of a day? Does morning mouth goo contain high levels of histamine, and should it be rinsed out? Are the colors in lipstick, like food coloring, off limits to masto patients? How about the petroleum base of many lipsticks and moisturizers?

    This has implications for other mast cell patients. I’ve looked all over the internet and cannot find answers, and my dr, whom I do respect, would probably make up the answers. If you’ve dealt with this in prior posts, just refer me.

    Again, thanks,
    Yvonne (ISM)

  2. Ashley Coats June 7, 2016 / 3:54 pm

    Could it also be that the heart/ventricle/mycardium fragility is attributed to a co-occurring diagnosis of Vascular Ehlers Danlos? From what I can surmise, Mast Cell and EDS often go hand in hand. Just a thought!

    • Lisa Klimas June 7, 2016 / 6:34 pm

      VEDS is definitely a consideration and can cooccur in mast cell patients. It has the benefit of having a known mutation associated so many patients get tested. Often the first sign of VEDS is rupture. We know with certainty because of animal models and human biopsies and testing that mast cell mediators can trigger the effects discussed in this series. Kounis Syndrome in particular is noteworthy for the fact that underlying vascular disease is often not present.

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